Beyond Dry Eyes: Connecting the Dots… Primary Sjogren’s Syndrome and Interstitial Lung Disease

Summary: Background: Primary Sjogren’s syndrome (pSS) affects mostly middle-aged women. Interstitial lung disease (ILD) is the most frequent lung abnormality prevalent in 20% of the pSS patients with a significant morbidity and mortality. Usual interstitial pneumonia (UIP) is prevalent in 10% of pSS patients who are older and mostly women. The UIP pattern is associated with pulmonary disease progression and unresponsive to immunosuppression treatment. Clinical examination is an insensitive tool to detect pSS-ILD. Complaints of fatigue, dyspnea and cough have a poor relationship with the presence of ILD. Case Presentation: A 79-year-old Caucasian male complaining of dry cough, dry eyes and dyspnea on exertion for three years was referred for autoimmune testing. PFT’s were consistent with restrictive lung disease. High-resolution CT scan of chest showed usual interstitial pneumonia (UIP). His ANA was 1:320, SSA and SSB negative, myositis panel negative, and protein serum electrophoresis negative. Schirmer tear test was abnormal with 2-3mm O.U. A salivary gland biopsy showed mixed type accessory salivary gland tissue with >3 focus of lymphocytic/plasmacytic-inflammation approximating >50 lymphocytes and plasma cells with normal architecture. He was diagnosed with Sjogren’s syndrome and started on mycophenolate mofetil. Discussion: Our case illustrates an elderly man with pSS-ILD UIP pattern who had SICCA symptoms for three years and needed the involvement of a rheumatologist to make the diagnosis. When ILD is diagnosed, Sjogren’s syndrome screening should be done. When Sjogren’s syndrome is diagnosed, ILD screening should be performed. Conclusions: The diagnosis of pSS-ILD is not easy due to a large number of asymptomatic or mildly symptomatic patients combined with the lack of specific biomarkers that leads to the progression of severe ILD in these patients. The recognition and improved understanding of the pathogenesis of pSS with the aid of rheumatologists will lead to earlier recognition and treatment of patients.